Dataset Information

Outcomes of Kidney Transplantation in Fabry Disease: A Meta-Analysis.

ABSTRACT:

Background

Fabry disease (FD) is a rare X-linked lysosomal storage disorder with progressive systemic deposition of globotriaosylceramide, leading to life-threatening cardiac, central nervous system, and kidney disease. Current therapy involves symptomatic medical management, enzyme replacement therapy (ERT), dialysis, kidney transplantation, and, more recently, gene therapy. The aim of this systematic review was to assess outcomes of kidney transplantation among patients with FD.

Methods

A comprehensive literature review was conducted utilizing MEDLINE, EMBASE, and Cochrane Database, from inception through to 28 February 2020, to identify studies that evaluate outcomes of kidney transplantation including patient and allograft survival among kidney transplant patients with FD. Effect estimates from each study were extracted and combined using the random-effects generic inverse variance method of DerSimonian and Laird.

Results

In total, 11 studies, including 424 kidney transplant recipients with FD, were enrolled. The post-transplant median follow-up time ranged from 3 to 11.5 years. Overall, the pooled estimated rates of all-cause graft failure, graft failure before death, and allograft rejection were 32.5% (95%CI: 23.9%-42.5%), 14.5% (95%CI: 8.4%-23.7%), and 20.2% (95%CI: 15.4%-25.9%), respectively. In the sensitivity analysis, limited only to the recent studies (year 2001 or newer when ERT became available), the pooled estimated rates of all-cause graft failure, graft failure before death, and allograft rejection were 28.1% (95%CI: 20.5%-37.3%), 11.7% (95%CI: 8.4%-16.0%), and 20.2% (95%CI: 15.5%-26.0%), respectively. The pooled estimated rate of biopsy proven FD recurrence was 11.1% (95%CI: 3.6%-29.4%), respectively. There are no significant differences in the risks of all-cause graft failure (p = 0.10) or mortality (0.48) among recipients with vs. without FD.

Conclusions

Despite possible FD recurrence after transplantation of 11.1%, allograft and patient survival are comparable among kidney transplant recipients with vs. without FD.

SUBMITTER: Suarez MLG

PROVIDER: S-EPMC7838795 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Publications

Outcomes of Kidney Transplantation in Fabry Disease: A Meta-Analysis.

Suarez Maria L Gonzalez MLG Thongprayoon Charat C Hansrivijit Panupong P Medaura Juan J Vaitla Pradeep P Mao Michael A MA Bathini Tarun T Boonpheng Boonphiphop B Kanduri Swetha R SR Kovvuru Karthik K Basu Arpita A Cheungpasitporn Wisit W

Diseases (Basel, Switzerland) 20201223 1

<h4>Background</h4>Fabry disease (FD) is a rare X-linked lysosomal storage disorder with progressive systemic deposition of globotriaosylceramide, leading to life-threatening cardiac, central nervous system, and kidney disease. Current therapy involves symptomatic medical management, enzyme replacement therapy (ERT), dialysis, kidney transplantation, and, more recently, gene therapy. The aim of this systematic review was to assess outcomes of kidney transplantation among patients with FD.<h4>Met ...[more]

PMID: 33374610

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Dataset Information

Outcomes of Kidney Transplantation in Fabry Disease: A Meta-Analysis.

Background

Methods

Results

Conclusions

Publications

Outcomes of Kidney Transplantation in Fabry Disease: A Meta-Analysis.

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